Ear Implants Research Articles

Status of early hearing detection and intervention in South Korea: a nationwide population-based study of national infant health checkup

The aim of this study was to evaluate the status of early hearing detection and intervention after newborn hearing screening (NHS) in South Korea. A retrospective review of Korean national health insurance service data of all infants receiving the 4-month old national infant health checkup between 2010 and 2016 from a nationwide population-based database was conducted. Based on the results of the NHS-administered hearing questionnaires as part of the national infant health checkup, individuals were classified into “pass” (1,730,615 infants) or “refer” (10,941 infants) groups. Next, an analysis was conducted of age and the frequencies of tracking audiologic tests and surgeries of the middle ear (ME) and cochlear implants (CI). Diagnostic auditory brainstem response and audiometry, and surgeries of ME and CI were significantly performed more and earlier in the refer group compared with the pass group. For infants in the pass group who were presumed to have delayed or acquired hearing loss, the time of the first audiology tests and CI surgery was significantly delayed compared to those in the refer group; the average ages for first CI were 37 and 52 months in the refer group and pass group, respectively. Therefore, for early detection of delayed-onset hearing loss, regular hearing screening programs should be considered throughout the preschool ages.

Editorial introductions

Editorial introductions

Cochlear Implants: Evaluation of Effects of Various Parameters on Outcomes in Pediatric Patients at a Tertiary Care Centre for Unilateral Ear Implantation.

To determine whether variables such as Age, Gender, Demographic background of the patient and Pre-operative usage of hearing aids affect the outcomes of pediatric cochlear implant surgery when modified; in terms of speech and hearing gain. A hospital based retrospective-prospective type of cohort study was conducted over a period of 5years at a Tertiary care Teaching hospital and referral centre covering a population of about 68.9 million. Candidates selected were 1-5years of age with bilateral congenital severe-profound sensori-neural hearing loss. 50 patients were selected and were operated using VERIA technique of Cochlear Implant Surgery. Intraoperative testing of electrode functioning was done in all patients using NRT technique. The switching on of implant was done after 1month, following which patients underwent 100 sessions of auditory verbal therapy and training. Outcomes were evaluated in terms of hearing and speech gain by using Revised CAP scores, ITMAIS scores and PEACH scores in the loco-regional language. Those implanted at a younger age and with at least 3months of hearing aid usage pre-operatively had better outcomes measures. There was no effect on outcomes when the gender and demographic origin of the patient were compared. Candidates implanted before 3years age give better results and they should be encouraged to use hearing aid regularly and continuously before the surgery and should be advised trial and fitting as soon as CI planning begins. Also, gender and demographic background should not be considered when planning CI as these have no significant effect on outcomes.

Intraoperative quantification of floating mass transducer coupling quality in active middle ear implants: a multicenter study

PurposeEvaluating the effectiveness of intraoperative auditory brainstem responses (ABRs) to stimulation by the Vibrant Soundbridge (VSB) active middle ear implant for quantifying the implant’s floating mass transducer (FMT) coupling quality.MethodsIn a diagnostic multicentric study, patients (> 18 years) who received a VSB with different coupling modalities were included. Pre- and postoperative bone conduction thresholds, intraoperative VSB-evoked ABR thresholds (VSB-ABR) using a modified audio processor programmed to preoperative bone conduction thresholds, postoperative vibrogram thresholds, and postoperative VSB-ABR thresholds were measured. Coupling quality was calculated from the difference between the pure tone average at 1000, 2000, and 4000 Hz (3PTA) vibrogram and postoperative 3PTA bone conduction thresholds.ResultsTwenty-three patients (13 males, 10 females, mean age 56.6 (± 12.5) years) were included in the study. Intraoperative VSB-ABR response thresholds could be obtained in all except one patient where the threshold was > 30 dB nHL. Postoperatively, an insufficient coupling of 36.7 dB was confirmed in this patient. In a Bland–Altman analysis of the intraoperative VSB-ABRs and coupling quality, the limits of agreement exceeded ± 10 dB, i.e., the maximum allowed difference considered as not clinically important but the variation was within the general precision of auditory brainstem responses to predict behavioral thresholds. Five outliers were identified. In two patients, the postoperative VSB-ABR thresholds were in agreement with the coupling quality, indicating a change of coupling before the postoperative testing.ConclusionThe response thresholds recorded in this set-up have the potential to predict the VSB coupling quality and optimize postoperative audiological results.

Biomechanics of the Human Middle Ear with Viscoelasticity of the Maxwell and the Kelvin-Voigt Type and Relaxation Effect.

The middle ear is one of the smallest biomechanical systems in the human body and is responsible for the hearing process. Hearing is modelled in different ways and by various methods. In this paper, three-degree-of-freedom models of the human middle ear with different viscoelastic properties are proposed. Model 1 uses the Maxwell type viscoelasticity, Model 2 is based on the Kelvin–Voigt viscoelasticity, and Model 3 uses the Kelvin–Voigt viscoelasticity with relaxation effect. The primary aim of the study is to compare the models and their dynamic responses to a voice excitation. The novelty of this study lies in using different models of viscoelasticity and relaxation effect that has been previously unstudied. First, mathematical models of the middle ear were built, then they were solved numerically by the Runge–Kutta procedure and finally, numerical results were compared with those obtained from experiments carried out on the temporal bone with the Laser Doppler Vibrometer. The models exhibit differences in the natural frequency and amplitudes near the second resonance. All analysed models can be used for modelling the rapidly changing processes that occur in the ear and to control active middle ear implants.

Feasibility and Efficacy of Vibrant Soundbridge Short Process Coupler in Patients With Aural Atresia.

Different options are available for hearing restoration in patients with aural atresia. Middle ear implantation with the Vibrant Soundbridge (VSB) has been used successfully in many patients with aural atresia with conductive or mixed hearing loss. The aim of the current study was to assess the safety and the efficiency of VSBs coupled to the short process of the incus in patients with aural atresia with conductive or mixed hearing loss. The study was a retrospective chart review conducted at a tertiary university hospital. Audiological and surgical data from six ears with aural atresia implanted with VSBs coupled to the short process of the incus were analyzed. The audiological results were compared with previously reported results obtained after the utilization of different coupling techniques. All six atretic ears were successfully implanted using the short process coupler, and there were no major complications. Postoperative bone conduction thresholds did not differ substantially from preoperative thresholds. The respective mean preoperative pure-tone average 4, speech reception threshold, and word recognition score values were 56.7 dB, 61.7 dB, and 44%. Postoperative pure-tone average 4 thresholds (25 ± 3.5 dB) and speech reception thresholds (20.8 ± 8 dB) were significantly improved. The maximum postoperative word recognition score achieved was 100%. The audiological outcomes obtained were similar to previously reported outcomes after classic stapes placement. Coupling of the floating mass transducer to the short process of the incus in patients with aural atresia resulted in significant audiological improvements that were comparable to previously reported improvements after placement via other methods.

Fully Implantable Active Middle Ear Implants After Subtotal Petrosectomy With Fat Obliteration: Preliminary Clinical Results.

In patients with chronic middle ear disease, especially after revision surgery for ventilation problems and mixed hearing loss, active middle ear implants may provide an alternative treatment option. The fully implantable active middle-ear implant (FI-AMEI) is designed for implantation in a ventilated mastoid with an intact posterior canal wall. Until now, there have been no reports on audiometric results after implantation of a FI-AMEI in a fat-obliterated cavity after subtotal petrosectomy (SPE). Retrospective case review. Tertiary referral center. Twelve patients were included after numerous previous tympanoplasty surgeries for severe mixed hearing loss and FI-AMEI implantation. In five patients, the FI-AMEI was implanted in a cavity, with fat obliteration, after SPE. Seven patients received FI-AMEI implantation after intact canal wall (ICW) surgery. Audiometric results (pure-tone audiometry, Freiburger monosyllables) are demonstrated for 12 patients after an observation period of 3 months. The improvement in monosyllable score was 40 to 85% for the 12 patients. Free-field-aided thresholds showed high heterogeneity. FI-AMEI implantation combined with SPE provides an alternative approach to hearing rehabilitation to non-FI-AMEI implantation. Studies with a high number of patients and long-term observation periods are necessary to statistically verify these results.

A Quantitative Approach for the Objective Assessment of Coupling Efficiency for an Active Middle Ear Implant by Recording Auditory Steady-state Responses.

The coupling efficiency of a semi-implantable active middle ear implant with an electromagnetically driven floating mass transducer coupled to a middle ear ossicle or the round window can only be quantified postoperatively in cooperative patients by measuring behavioral vibroplasty in situ thresholds in comparison with bone conduction thresholds. The objective of the study was to develop a method to objectively determine the vibroplasty in situ thresholds by determining calibration factors from the relation between the objective and behavioral vibroplasty in situ thresholds. Prospective experimental study. Fifteen patients, implanted with an active middle ear implant for at least 6 months. Diagnostic. Vibroplasty in situ thresholds measured objectively by auditory steady state responses (floating mass transducer [FMT]ASSR). Subjective vibroplasty in situ thresholds were measured in this experimental ASSR set-up (FMTsubj.) and behaviorally by pure-tone audiometry (vibrogram). All thresholds were obtained at 500, 1000, 2000, and 4000 Hz. Thresholds could be objectively measured by ASSR in all patients. Differences between the FMTASSR and FMTsubj. were statistically significant but small. A significant correlation was found between the FMTASSR and the vibrogram thresholds at 4000 Hz but not for the other test frequencies. The method is feasible to measure objective vibroplasty in situ thresholds in active middle ear implant users. Since calibration factors could not be determined for all frequencies, the method is only applicable to comparatively determine the coupling efficiency of the floating mass transducer.

Operative techniques in Otolaryngology: Hearing restoration options

Hearing loss in elderly patients, commonly referred to as age-related hearing loss (ARHL), is an increasingly significant cause of morbidity. ARHL noted to be the second most common illness in the elderly population, and can often be detrimental to the quality of life of patients. Presently, restorative treatment for age-related hearing loss is limited, however, multifactorial rehabilitation and management strategies exist. A pivotal aspect of management in ARHL is the use of amplifying hearing aids and implantable devices. At this time, options for amplification include external and internal hearing aids, middle ear implants, bone-anchored devices, and cochlear implants. Despite the presence of numerous management options, many patients with hearing loss are not using amplifying devices. This chapter discusses the different management options for ARHL including important considerations and operative techniques, with cochlear implants being discussed in a separate chapter.

Effect of stimulation sites on the performance of electromagnetic middle ear implant: A finite element analysis

Electromagnetic middle ear implants (MEIs), which use the mechanical vibration of their implanted transducers to treat hearing loss, have emerged to overcome the limitations of conventional hearing aids. Several reports have indicated that the electromagnetic MEI's performance changed with different stimulation sites of the transducer. The aim of this study was to analyze the influence of the transducers' stimulation sites on the electromagnetic MEIs' performance. To aid this investigation, a human ear finite-element model was developed from micro-CT images of an adult's right ear. The validity of the model was confirmed by comparing the model-derived results with experimental data. Then, stimulation forces, which simulate ideal electromagnetic transducers, were respectively applied at five typical coupling sites: the umbo, incus body, incus long process, the round window, and the stapes. The stimulation sites' influence on the electromagnetic MEI's performance was studied by analyzing their corresponding basilar membrane displacements. The results show that stimulation of the round window with a force produces more cochlear stimulation than equal force stimulation of the umbo, incus body, incus long process and the stapes, though the superiority of the round window depends on its smaller area compared to the stapes footplate. Among the forward stimulation, the stapes is the optimal stimulation site for the electromagnetic transducer regarding its hearing compensation's efficiency. The performance of the umbo stimulation is comparable to that of the incus-long-process stimulation. Driving the incus body is less efficient than stimulating the other forward driving sites. Additional, using the stapes response to evaluate the forward stimulation gives results similar to those deduced by the basilar membrane response; in contrast, for the round-window stimulation, the evaluation result based on the stapes response is prominently less than the one calculated by the basilar membrane response, especially in the mid-high frequency range.

Subjective Hearing Impression and Quality of Life in Patients With Bilateral Active Middle Ear Implants.

This study compared the quality of life in patients with bilateral active middle ear implants to their quality of life when they were unilaterally implanted. Twenty-one patients implanted sequentially with the Vibrant Soundbridge (VSB) active middle ear implant completed quality-of-life surveys. The patients were asked to rate whether and to what extent their quality of life has changed upon receiving a second VSB. Hearing-specific quality of life was assessed with the Speech, Spatial, and Qualities of Hearing Scale (SSQ12-B), and general quality of life was with the Glasgow Benefit Inventory (GBI). In addition, the patients completed a health-related quality-of-life questionnaire (AQoL-8D). Finally, the changes in hearing-related and general quality of life were correlated to the change in word recognition ability after implantation of the second VSB (Freiburg monosyllabic word test; unilateral VSB versus bilateral VSB at 65 dB SPL). On the SSQ12, subjects scored a median of + 2.73 (p < 0.001; significantly different from zero) on a scale of -5 to + 5 where 0 indicates no change. All three subscores showed significant improvement. On the GBI, patients reached a median overall score of + 23.6 (p < 0.001; significantly different from zero) on a scale of -100 to + 100 where 0 indicates no change. Here, the improvement was mainly visible in the general subscore, whereas the social support and physical health subscores did not change due to the intervention. Both subjective benefit rating scores strongly correlated with the change in word recognition scores, suggesting that both hearing and general quality of life improved with increased word recognition due to bilateral VSB use. No significant correlation was found between the subjects' general health (as measured by AQoL-8D utility scores) and SSQ12-B or GBI overall scores. Usage of a second active middle ear implant substantially improved our patients' subjective hearing and general quality of life compared with unilateral use. The increase in quality of life may be linked to improved speech understanding due to bilateral use of a middle ear implant. Furthermore, these outcomes were not influenced by our patients' general health state at the time of survey.

Stability of the standard incus coupling of the Carina middle ear actuator after 1.5T MRI.

Limited data is available concerning the safety of active middle ear implants (AMEI) during Magnetic Resonance Imaging (MRI). Measurements in temporal bones are the gold standard for preclinical assessment of device safety. In this study the coupling stability of an actuator as used in a fully implantable AMEI was determined in temporal bones. Eleven temporal bones were implanted with the actuator according to the manufacturer's surgical guidelines. The actuator was coupled on the incus short process as recommended for sensorineural hearing loss. Temporal bones were exposed 10 times to the MRI magnetic field by entering the MRI suite in a clinically relevant way. Computed Tomography (CT) images were acquired before and after the experiment to investigate the risk of actuator dislocation. Based on the electrical impedance of the actuator, the loading of the actuator to the incus was confirmed. Relative actuator displacement was determined on the CT images by comparing the initial with the final actuator position in 3D space. Impedance curves were analyzed after each exposure to check the loading of the actuator to the ossicles. Analysis of CT images with a 0.30.6 mm in-plane resolution indicate no actuator displacement. The maximum detected change in impedance for all actuators was 8.43 Ω at the actuator's resonance frequency. Impedance curves measured when the actuator was retracted from the short process after the experiment still indicate the presence of a clear resonance peak. No actuator displacement or dislocation could be detected in the analysis of CT images and the measured impedance curves. Impedance curves obtained when the actuator was retracted from the incus short process still show a clear resonance peak, indicating the device is still functional after the MRI exposures.

Minimally-invasive incision using the implantable Hearing device Carina

The skin incision for cochlear implants and active middle ear implants like Vibrant Soundbridge and Bonebridge has evolved toward a minimally invasive access in recent years.For the active middle ear implant CARINA a significantly larger incision is recommended, which corresponds to that of Cochlear Implant care nearly two decades ago.As a result, increased complication rates due to extended incision can be expected. In order to minimize the complication rate for the active middle ear implant CARINA the possibility for minimally invasive incisions was reconsidered and developed. Up to now, 4 patients have been successfully treated with a CARINA system with the minimally invasive skin incision of less than 5 cm length without postoperative complications. By optimizing the fixation by using self-tapping screws, this access route can be established as a standard.

Location of the stapedius muscle with reference to the facial nerve in patients with unilateral congenital aural atresia: implication for active middle ear implants surgery

Background: Detailed investigations of the stapedial muscle (SM) in congenital aural atresia (CAA) patients have yet to be adequately conducted.Objectives: To assess image variations in the mastoid segment of the facial nerve (FN) and SM in CAA.Materials and methods: A total of nine patients comprising of 9 ears with unilateral CAA were studied. The courses of the FN and SM were evaluated from the basic point to 1 mm intervals between the mastoid portion of FN, and measured from the mean X and Y values in each group.Results: The atresia side of FN among the Y values showed significant differences compared to the contralateral side. In terms of the SM, there were no significant differences in both the X and Y values. The stapedial muscle of the CAA patients was located medially to the FN. Conversely, the distance from the PSC to the FN revealed no significant differences with regard to the X and Y values for each group.Conclusion: The current observations revealed that the SM is located more posterior to the FN in CAA patients, and this is mainly attributed to the laterally and anteriorly displaced FN.

Hearing Aid Treatment in Patients with Mixed Hearing Loss. Part I: Expected Benefit and Limitations after Stapes Surgery

Objective: The purpose of the present study was to determine the fraction of patients with mixed hearing loss who can or cannot expect benefit from power hearing aids (HAs) after stapes surgery. Design: The audiological outcome of 374 stapes surgeries was used to calculate the patients’ individual postoperative requirements in terms of gain and output of HAs. These requirements were compared to the available gain and output provided by state-of-the-art power HAs at 0.5, 1.0, 2.0, and 4.0 kHz. According to these comparisons, ears were divided into three groups. For G0, required gain and output lay within the corresponding technical limits of the HAs at all frequencies. In G1, one or both requirements could not be fulfilled at 1 frequency. G2 combined all ears where the requirements lay beyond the HA’s technical limitations at 2 or more frequencies. Results: Stapes surgery resulted in an improvement of air-bone gap (ABG) in 84.5% of the cases by 15.7 dB on average. Based on pure-tone average (0.5, 1.0, 2.0, 4.0 kHz), 40.6% of all cases showed an ABG ≤10 dB. 44.9% of all cases did no longer need a HA after stapes surgery. A power HA would fulfill both audiological criteria at all 4 frequencies in 81.6% of cases that needed a HA postoperatively. However, 18.4% would not be sufficiently treatable at 1 or more frequencies (15.0% in G1, 3.4% in G2). Conclusions: The present study identified a subset of patients with mixed hearing loss after stapes surgery that cannot be treated sufficiently with available power HAs. As the residual ABG is an important reason for this lack of treatment success, the advancement of alternative hearing devices that circumvent the middle ear, such as powerful active middle ear implants, is indicated.

Effects of Vibrant Soundbridge on tinnitus accompanied by sensorineural hearing loss.

Tinnitus is a common symptom among patients with hearing loss, and many studies have reported successful tinnitus suppression with hearing devices. Active middle ear implantation of the Vibrant Soundbridge (VSB) is a good alternative to existing hearing devices. This study evaluated the effects of VSB implantation on tinnitus and sought to identify the main audiological factor that affects tinnitus suppression. The study participants were 16 adults who had tinnitus with sensorineural hearing loss, and who underwent VSB implantations. Pure-tone audiometry; word recognition test; tinnitus handicap inventory (THI); and visual analog scale (VAS) assessment of loudness, awareness, and annoyance were performed before and 12 months after surgery. Changes in hearing threshold, word recognition scores (WRS), THI scores, and VAS scores were analyzed. VAS scores for loudness (mean difference: 1.9, 95% CI: 0.6, 3.1), awareness (mean difference: 1.6, 95% CI: 0.4, 2.8), and annoyance (mean difference: 1.7, 95% CI: 0.7, 2.8) showed significant improvements from baseline to 12 months after surgery. In addition, THI scores showed a significant decrease (mean difference: 13.8, 95% CI: 2.9, 24.9). The average hearing threshold level, WRS, and most comfortable level (MCL) also showed significant improvements at 12 months after surgery (mean difference: 17.3, 95% CI: 13.3, 21.3; mean difference: -7.6, 95% CI: -15.1, -0.1; mean difference: 26.3, 95% CI: 22.9, 29.6, respectively). Among the aforementioned factors, changes in MCL were best correlated with those in THI scores (mean difference: 2.55, 95% CI: 0.90, 4.21). A VSB implant is beneficial to subjects with tinnitus accompanied by sensorineural hearing loss. The changes in THI scores best correlated with those in MCL. This improvement may represent a masking effect that contributes to tinnitus suppression in patients with VSB implants.

A Precision Driver Device for Intraoperative Stimulation of a Bone Conduction Implant

Semi-implantable bone conduction implants (BCI) and active middle ear implants (AMEI) for patients with sensorineural, conductive or mixed hearing loss commonly use an amplitude modulation technology to transmit power and sound signals from an external audio processor to the implant. In patients, the distance dependence of the signal amplitude is of minor importance as the skin thickness is constant and only varies between 3–7 mm. In this range, critical coupling transmission technique sufficiently reduces the variability in amplitude, but fails to provide well-defined amplitudes in many research and clinical applications such as intraoperative integrity tests where the distance range is exceeded by using sterile covers. Here we used the BCI Bonebridge (BB, Med-El, Austria) as an example to develop and demonstrate a system that synthesizes the transmission signal, determines the distance between the transmitter and the receiver implant coil and compensates transmission losses. When compared to an external audio processor (AP304) on an artificial mastoid, our system mainly decreased amplitude variability from over 11 dB to less than 3 dB for audio frequencies (0.1–10 kHz) at distances up to 15 mm, making it adequate for intraoperative and audiometric tests.

Near-infrared excitation of nitrogen-doped ultrananocrystalline diamond photoelectrodes in saline solution

Nitrogen-doped ultrananocrystalline diamond (N-UNCD) is a promising material for a variety of neural interfacing applications, due to its unique combination of high conductivity, bioinertness, and durability. One emerging application for N-UNCD is as a photoelectrode material for high-precision optical neural stimulation. This may be used for the treatment of neurological disorders and for implantable bionic devices such as cochlear ear implants and retinal prostheses. N-UNCD is a well-suited material for stimulation photoelectrodes, exhibiting a photocurrent response to light at visible wavelengths with a high charge injection density [A. Ahnood, A. N. Simonov, J. S. Laird, M. I. Maturana, K. Ganesan, A. Stacey, M. R. Ibbotson, L. Spiccia, and S. Prawer, Appl. Phys. Lett. 108, 104,103 (2016)]. In this study, the photoresponse of N-UNCD to near-infrared (NIR) irradiation is measured. NIR light has greater optical penetration through tissue than visible wavelengths, opening the possibility to stimulate previously inaccessible target cells. It is found that N-UNCD exhibits a photoresponsivity which diminishes rapidly with increasing wavelength and is attributed to transitions between mid-gap states associated with the graphitic phase present at the grain boundaries and the conduction band tail. Oxygen surface termination on the diamond films provides further enhancement of the injected charge per photon, compared to as-grown or hydrogen terminated surfaces. Based on the measured injected charge density, we estimate that the generated photocurrent of oxygen terminated N-UNCD is sufficient to achieve extracellular stimulation of brain tissue within the safe optical exposure limit.

Active middle ear implantation: imaging in the pre-operative planning and post-operative assessment of the Vibrant SoundbridgeTM.

Active middle ear implants augment sound waves and directly stimulate the middle ear structures. The most frequently utilised active middle ear implant is the Vibrant Soundbridge TM (VSB).CT plays a vital role in appropriate patient selection and surgical planning of active middle ear implant surgery. The VSB TM offers a number of options for implant placement. The ideal location is influenced by the patient's middle ear and mastoid anatomy as well as the type and severity of the hearing loss. CT provides important information on the surgical access to the middle ear and helps determine the most appropriate implant site by assessing the adjacent middle ear anatomy and the continuity of the ossicular chain. Post-operative active middle ear implant imaging may be indicated in the setting of poor auditory outcomes and when revision surgery is being considered so as to assess for suboptimal implant placement or migration.This pictorial review will describe the VSB TM middle ear device and explain the role of imaging in both the pre-operative and post-operative settings.

Genetic Inheritance of Late-Onset, Down-Sloping Hearing Loss and Its Implications for Auditory Rehabilitation.

Late-onset, down-sloping sensorineural hearing loss has many genetic and nongenetic etiologies, but the proportion of this commonly encountered type of hearing loss attributable to genetic causes is not well known. In this study, the authors performed genetic analysis using next-generation sequencing techniques in patients showing late-onset, down-sloping sensorineural hearing loss with preserved low-frequency hearing, and investigated the clinical implications of the variants identified. From a cohort of patients with hearing loss at a tertiary referral hospital, 18 unrelated probands with down-sloping sensorineural hearing loss of late onset were included in this study. Down-sloping hearing loss was defined as a mean low-frequency threshold at 250 Hz and 500 Hz less than or equal to 40 dB HL and a mean high-frequency threshold at 1, 2, and 4 kHz greater than 40 dB HL. The authors performed whole-exome sequencing and segregation analysis to identify the genetic causes and evaluated the outcomes of auditory rehabilitation in the patients. There were nine simplex and nine multiplex families included, in which the causative variants were found in six of 18 probands, demonstrating a detection rate of 33.3%. Various types of variants, including five novel and three known variants, were detected in the MYH14, MYH9, USH2A, COL11A2, and TMPRSS3 genes. The outcome of cochlear and middle ear implants in patients identified with pathogenic variants was satisfactory. There was no statistically significant difference between pathogenic variant-positive and pathogenic variant-negative groups in terms of onset age, family history of hearing loss, pure-tone threshold, or speech discrimination scores. The proportion of patients with late-onset, down-sloping hearing loss identified with potentially causative variants was unexpectedly high. Identification of the causative variants will offer insights on hearing loss progression and prognosis regarding various modes of auditory rehabilitation, as well as possible concomitant syndromic features.